Figure 4.
NPC1/2 expression correlates with degree of alexidine-induced lysosomal cholesterol reduction. (A) Control, patient NPC1I1061T/I1061T, NPC1−/− and NPC2−/− fibroblasts were treated with DMSO (0.05%), 122 nm alexidine, or 1.1 μm alexidine for 72 h and then fixed, stained with filipin and anti-LAMP1 and imaged. Quantification of filipin (A) and LAMP1 (B) and representative images of treated cells (C). Scale bar = 50 μM. Bars: mean ± SD, n = 4 wells/condition, ****P < 0.0001, ***P < 0.001, **P < 0.01 versus genotype-matched DMSO, alexidine two-way ANOVA followed by Dunnett’s test.